Dercum's Disease: A Quick Overview

This overview was put together to tie into the current fundraiser.  If you can donate, please donate and forward on the word.  If you can't donate, please pass this on to family and friends.  Thanks.

Chronic Pain Stats

 Chronic Pain is one of Dercum's worst symptoms.The info is staggering and yet most are still not getting the relief from the pain.

Short article about lipedema

If you are like me and have both Dercum's AND Lipedema, here is a short article you will appreciate.

International leader in lipedema management stresses need for greater awareness

Publish date: Dec 1, 2011
By: Cheryl Guttman KraderSource: Cosmetic Surgery Times

• Women are affected by lipedema, which most often develops in the upper legs
• Abnormal fat that accumulates in lipedema is resistant to conventional weight-loss measures
• Early intervention can help to prevent progression

Liposuction using super tumescent local anesthesia is an important advance for the safe and effective treatment of lipedema, but appropriate diagnosis is the first step to successful management of this disorder, which causes significant morbidity and is more common than generally recognized, says Louis Habbema, M.D., Ph.D.

Dr. Habbema

In lipedema, there is abnormal accumulation of subcutaneous fat that can be accompanied by mild edema in the extremities. Women, almost exclusively, are affected by the condition, which occurs bilaterally and symmetrically, and most often develops in the upper legs. The lower legs and upper arms may be involved, but lipedema rarely affects the lower arms, and fat accumulation in lipedema never occurs in the trunk or face.
Some patients develop lymphedema as well, probably due to compression of the lymph vessels. Lipedema patients also may show anatomically abnormal lymph vessels, Dr. Habbema says.

Due to a low awareness of the condition among physicians, patients seeking help may simply be counseled to lose weight and/or offered liposuction using conventional techniques. Although women with lipedema may also suffer from obesity, the abnormal fat that accumulates in lipedema is very resistant to conventional weight-loss measures, and it is destined to accumulate again after removal with conventional liposuction, says Dr. Habbema, who has been at the forefront of developing treatment for lipedema and efforts to increase awareness about the condition.
"Fat accumulation in lipedema cannot be controlled or eliminated through diet, and the swelling and aching associated with the condition are worsened by exercise. Women attempting to lose weight by these methods will simply lose fat from areas of the body that are not affected by lipedema, including the face, the breasts and the trunk, while the lipedema gets progressively worse," says Dr. Habbema, a dermatologist specializing in cosmetic medicine and founder, Medical Centre 't Gooi, Amsterdam.
"Our experience with around 800 women shows that lipedema treatment with circumferential liposuction performed using a large volume of tumescent anesthesia safely and effectively removes the fat and improves patient symptoms," he says. "Early intervention is desired to hopefully prevent progression and improve quality of life. However, the doctor must first recognize the problem in order to offer the correct treatment."

ASSOCIATED MORBIDITY The morbidity associated with lipedema includes physical, psychosocial and functional consequences. In addition to having low self-esteem because of their appearance, affected women become frustrated and discouraged by the failure of their weight-loss efforts, and the psychological burden may be compounded for those who go from physician to physician seeking help, only to receive the same advice about diet and exercise that they have already tried and know doesn't work.

In severe cases, the enlarged extremities can cause mechanical sequelae, including mobility issues and friction-induced skin breakdown. Expansion of the subcutaneous compartment with fat also causes discomfort, tenderness or even severe pain, and the affected areas may be prone to easy bruising.

"Women who've enjoyed long walks may find they must stop after just 10 minutes because of their pain, and merely touching the surface of the skin can cause distress," Dr. Habbema says.

"In addition, the pain may be misdiagnosed as originating from some other musculoskeletal condition and lead women to undergo unnecessary diagnostic and surgical procedures that will have no benefit on their chief complaint."

Interesting Findings

Mechanisms driving fat accumulation in Dercum's disease investigated

Researchers using gas chromatography and mass spectrometry have found differences in desatrase activity among sufferers of Dercum’s disease (DD), compared with obese control subjects and subjects with familial multiple lipomatosis (FML).

The research opens up new avenues for exploration in relation to desaturation index (DI) of DD, multiple symmetric lipomatosis (MSL) and FML, providing further information on adipose tissue fatty acid metabolism in these disorders. The potential relationship between the DI and adipokines or adiposity measures in obesity and Reactive Airways Dysfunction Syndrome (RADs) may now be explored.

Researchers obtained subcutaneous adipose tissue from human participants with DD, MSL,FML and obese controls and determined fatty acid composition by gas chromatography and mass spectrometry. The DIs (palmitoleic/palmitic, oleic/stearic, vaccenic/stearic ratios) were calculated from the gas chromatogram peak intensities. Finally, Spearman's correlations between the DIs and available clinical or biochemical data were performed.

The results showed that in DD subjects, the vaccenic and stearic index was lower in comparison to Controls. The saturated fatty acid myristic acid was higher in DD compared with Controls and FML, and the per cent of monounsaturated vaccenic acid in DD trended lower when compared with Controls, and was decreased in comparison to FML.

Posted by Fiona Griffiths

From Chromotography Today, Feb. 6, 2012

Two new articles by Dr. Karen Herbst

Dr. Herbst has been busy! There are two new articles on PubMed (go to www.pubmed.com) about Dercum's Disease and the other RADs. Please check them out.

And THANK YOU, Dr. Herbst, for all you do for us!!!

1. Rare adipose disorders (RADs) masquerading as obesity.
Herbst KL.
... Acta Pharmacol Sin. 2012 Feb;33(2):155-72. doi: 10.1038/aps.2011.153.
PMID:22301856[PubMed - in process]
If you go to the website this paper should be availabel for free download.

2.Subcutaneous adipose tissue fatty acid desaturation in adults with and without rare adipose disorders.
Yee JK, Phillips SA, Allamehzadeh K, Herbst KL.
Lipids Health Dis. 2012 Feb 3;11(1):19. [Epub ahead of print]
PMID:22300160[PubMed - as supplied by publisher] Free ArticleSee More

Dercum's Disease Symptoms

Always worth repeating if you're just checking our site. -

Dercum's Disease Symptoms

If you have found this site because you are trying to find out what is wrong with you, here is a good list of symptoms. Note that not everybody has every symptom. But a surprising number of people with Dercum's Disease have many of them. If you're like most of us, you will read the list and be thinking "Uh-huh", "Oh yeah!", "Oh that explains THAT!"

I hope this will help you on your way to finding an answer!

• Presentation
  • Previously healthy women notice lumps or previously present lumps start growing. They describe pain and discomfort in the region of the lumps associated with weakness.
  • Before the onset of the disease, the patient is usually only slightly obese, but, in a short time, the patient becomes overweight.
  • The pain increases with the increase in fatty tissue and in connection to menstruation.
• Types and location
  

  • The painful lipomas have been reported to occur in any location, except in the head and the neck. ^{(Our note - they can also appear there.)}
  • Different types can be identified according to the spread of pain.
    • Type I, or the juxta-articular type, with painful folds of fat on the inside of the knees and/or on the hips, in rare cases only evident in upper-arm fat
    • Type II, or the diffuse, generalized type, where widespread pain from fatty tissue is found, apart from that of type I, also often in the dorsal upper-arm fat, in the axillary and gluteal fat, in the stomach wall, in dorsal fat folds, and on the soles of the feet
    • Type III, or the lipomatosis, nodular type, with intense pain in and around multiple lipomas, sometimes in the absence of general obesity; lipomas are approximately 0.5-4 cm, soft, and attached to the surrounding tissue (Histologically, these are not always encapsulated. Some have been classified as angiolipomas.)
• Type of pain
  

  • The pain varies from discomfort on palpation to excruciating, paroxysmal spontaneous attacks.
  • The pain can be aching, burning, or stabbing, often described by the patient as "it hurts everywhere."
  • The pain is usually symmetrical; however, it can become localized to the thighs, the knees, or the upper extremities.
  • Pain can be felt in the skeletal system and in the fat.
• Hyperalgesia is found by light pressure and touch in the fatty tissue below the skin and is made worse by tightly fitting clothes or showering. The pain is temperature and weather dependent; it decreases in dry heat and when pressure is high. Hot baths can have a positive but short-term effect in the relief of pain, but some patients do not tolerate heat. Estrogen replacement at menopause does not reduce the pain.
• Other symptoms, with variable incidence, include the following:
  • The fingers have a tendency to swell up, fumble, and tingle, and they can be numb (paresthesias), in addition to secondary median nerve compression.
  • General tiredness similar to the symptoms of chronic fatigue syndrome may be present. Light physical activity and poor sleep aggravate the tiredness.
  • A tendency to bruise, possibly secondary to the formation of delicate vessels in fat deposits, may be present. Coagulation test results are normal.
  • Morning stiffness and stiffness after resting may occur.
  • Headaches (eg, tension headaches, classic migraine, neck headaches) may occur. Also, pain in the jaw and the eyes due to retrobulbar fatty tissue may be present.
  • Cognitive dysfunction, with concentration and memory problems, may be present.
  • Bouts of depression (atypical depression, possibly latent) may occur; this finding is not associated with the onset of the disease.
  • Feeling hot affects a small number of patients, with recurring high temperatures of 37.5-39°C for weeks at a time associated with worsening of pain.
  • Patients may become susceptible to infection, which is possibly due to the presence of fat. Pain is exacerbated with infections.

Physical

• Dercum disease (adiposis dolorosa) symptoms are almost always out of proportion to the physical findings, which include the following:
  • Dercum disease (adiposis dolorosa) patients are usually 50% over the normal weight for their age. In some patients, only localized fat, without general obesity, is present.
  • Lipomas are multiple, painful, symmetrically distributed, fatty deposits that are either diffuse or localized. The abdominal region and the lower extremities are common sites, especially around the knees. The ankle is an uncommon site of involvement.
  • Hyperalgesia is found in the fatty tissue below the skin on light pressure and touch.
  • Other findings include acral swelling, bruises, and telangiectasias.

The above information is an exerpt from the Adiposis Dolorosa article on Emedicine at http://emedicine.medscape.com/article/1082083-overview

Dercum's Disease Symptoms

If you have found this site because you are trying to find out what is wrong with you, here is a good list of symptoms. Note that not everybody has every symptom. But a surprising number of people with Dercum's Disease have many of them. If you're like most of us, you will read the list and be thinking "Uh-huh", "Oh yeah!", "Oh that explains THAT!"

I hope this will help you on your way to finding an answer!

• Presentation
  • Previously healthy women notice lumps or previously present lumps start growing. They describe pain and discomfort in the region of the lumps associated with weakness.
  • Before the onset of the disease, the patient is usually only slightly obese, but, in a short time, the patient becomes overweight.
  • The pain increases with the increase in fatty tissue and in connection to menstruation.
• Types and location
  

  • The painful lipomas have been reported to occur in any location, except in the head and the neck. ^{(Our note - they can also appear there.)}
  • Different types can be identified according to the spread of pain.
    • Type I, or the juxta-articular type, with painful folds of fat on the inside of the knees and/or on the hips, in rare cases only evident in upper-arm fat
    • Type II, or the diffuse, generalized type, where widespread pain from fatty tissue is found, apart from that of type I, also often in the dorsal upper-arm fat, in the axillary and gluteal fat, in the stomach wall, in dorsal fat folds, and on the soles of the feet
    • Type III, or the lipomatosis, nodular type, with intense pain in and around multiple lipomas, sometimes in the absence of general obesity; lipomas are approximately 0.5-4 cm, soft, and attached to the surrounding tissue (Histologically, these are not always encapsulated. Some have been classified as angiolipomas.)
• Type of pain
  

  • The pain varies from discomfort on palpation to excruciating, paroxysmal spontaneous attacks.
  • The pain can be aching, burning, or stabbing, often described by the patient as "it hurts everywhere."
  • The pain is usually symmetrical; however, it can become localized to the thighs, the knees, or the upper extremities.
  • Pain can be felt in the skeletal system and in the fat.
• Hyperalgesia is found by light pressure and touch in the fatty tissue below the skin and is made worse by tightly fitting clothes or showering. The pain is temperature and weather dependent; it decreases in dry heat and when pressure is high. Hot baths can have a positive but short-term effect in the relief of pain, but some patients do not tolerate heat. Estrogen replacement at menopause does not reduce the pain.
• Other symptoms, with variable incidence, include the following:
  • The fingers have a tendency to swell up, fumble, and tingle, and they can be numb (paresthesias), in addition to secondary median nerve compression.
  • General tiredness similar to the symptoms of chronic fatigue syndrome may be present. Light physical activity and poor sleep aggravate the tiredness.
  • A tendency to bruise, possibly secondary to the formation of delicate vessels in fat deposits, may be present. Coagulation test results are normal.
  • Morning stiffness and stiffness after resting may occur.
  • Headaches (eg, tension headaches, classic migraine, neck headaches) may occur. Also, pain in the jaw and the eyes due to retrobulbar fatty tissue may be present.
  • Cognitive dysfunction, with concentration and memory problems, may be present.
  • Bouts of depression (atypical depression, possibly latent) may occur; this finding is not associated with the onset of the disease.
  • Feeling hot affects a small number of patients, with recurring high temperatures of 37.5-39°C for weeks at a time associated with worsening of pain.
  • Patients may become susceptible to infection, which is possibly due to the presence of fat. Pain is exacerbated with infections.

Physical

• Dercum disease (adiposis dolorosa) symptoms are almost always out of proportion to the physical findings, which include the following:
  • Dercum disease (adiposis dolorosa) patients are usually 50% over the normal weight for their age. In some patients, only localized fat, without general obesity, is present.
  • Lipomas are multiple, painful, symmetrically distributed, fatty deposits that are either diffuse or localized. The abdominal region and the lower extremities are common sites, especially around the knees. The ankle is an uncommon site of involvement.
  • Hyperalgesia is found in the fatty tissue below the skin on light pressure and touch.
  • Other findings include acral swelling, bruises, and telangiectasias.

The above information is an exerpt from the Adiposis Dolorosa article on Emedicine at http://emedicine.medscape.com/article/1082083-overview

 

If you found this information helpful, please consider making a contribution to our fundraiser so that we can provide Dr. Herbst with funds to do additional Dercum's research. Thanks!

Brain Fog, Can It Be Helped?

Brain fog is the term used for the symptom of poor short term memory, lack of concentration, difficulty learning new things and my all time favorite, inability to think of or say the correct words.  This is a common symptom of long term illness and one of the many I have heard sufferers of Dercum’s complain about.

There is a plethora of reasons why people with Rare Adipose Disorders may suffer from brain fog:  low oxygen levels;  nutritional imbalances; elevated copper levels and other toxic metals; poor digestion; thyroid issues; hyperglycemia; allergies; and of course some of the medicines we consume to alleviate other symptoms of our disease.

The question is:  Can this be treated? 

Just like any other muscle in your body, your brain needs to be exercised.  As the saying goes, use it or lose it.  Mindfulness exercises, such as memory games, interaction with others and meditation can help stave off the brain fog.  Breathing exercises can also help by providing the body with much needed oxygen.  Studies have shown that Omega-3 Fish Oil and Vitamin D3 may help cognitive performance as well as help with overall well-being.  As with everything, nothing works overnight but by taking care of your brain with some or all of these suggestions, you may notice a difference over time.  

If your brain fog is severe, talk to your doctor about medication that may help. There are medications that are currently used for Narcolepsy, Obstructive Sleep Apnea, Shift Work Disorder and other known sleep disorders.  The class of drug is eugeroics, which are stimulants that provide long-lasting mental arousal.  I do admit this sounds fabulous but like any medication there are side effects to consider and with this class of drug being so new to the market in the USA (created in 1998 with Provigil), it is not always easy for a doctor to prescribe.  You may have to prove you have a sleep disorder first by going through a sleep study or two (it took me 3 times) before they will take out their little pad and write it up for you, or, more realistically today, type it into a computer and send it off to your pharmacy.

My suggestion is for 10 to 15 minutes every day, turn off the television, put your virtual farm and pets on hold and log into the many sites that have free “Brain Games”. There you can work on building your memory and concentration skills.  While you are clicking around, you may as well try to do something to improve your brain function before you forget to do it all together.  Here's to a more focused tomorrow!